Plasma exchange removes glycosphingolipid in Fabry disease.
In a man with Fabry disease, basal plasma glycosphingolipid (GSL) levels were determined by high-performance liquid chromatography (HPLC). A series of three alternate-day plasma exchanges transiently lowered plasma ceramide trihexoside (CTH) to normal. A total of 70 mu moles of CTH were removed by eight plasma exchanges. If future studies show that pathologic tissue accumulations of CTH are reduced by plasma exchange, then long-term repetitive plasma exchange could be used as treatment until enzyme replacement is practical.
D000328:Adult; D002851:Chromatography, High Pressure Liquid; D000795:Fabry Disease / Q000097:blood / Q000628:therapy*; D006028:Glycosphingolipids / Q000097:blood*; D006801:Humans; D008297:Male; D010951:Plasma Exchange; D014281:Trihexosylceramides / Q000097:blood*
D006028:Glycosphingolipids; D014281:Trihexosylceramides